Idiopathic Osteonecrosis Research Articles

The idiopathic avascular osteonecrosis of the third metacarpal head (M. Mauclaire/Dieterich's disease)

The long-term results of the vascularized bone graft of iliac bone in idiopathic osteonecrosis of the femoral head

Osteonecrosis is a debilitating toxicity associated with acute lymphoblastic leukemia (ALL) treatment. A recent report associated interindividual differences in hip anatomy with the development of idiopathic osteonecrosis in adults. To evaluate the impact of hip anatomy on the development of therapy-related osteonecrosis, we retrospectively evaluated the femoral neck-shaft angle, femoral neck offset, and lateral center-edge angle using x-rays of 18 osteonecrosis cases and 46 control children treated for newly diagnosed ALL on a single protocol. Despite adequate statistical power, we found no association between hip anatomy and osteonecrosis. Investigation of other factors contributing to ALL-associated osteonecrosis is warranted.

Osteonecrosis of the femoral head (ONFH) is a multifactorial disease and is associated with genetic predisposition, and exposure to certain risk factors. In particular, idiopathic ONFH in twins and the clustering of cases in families have indicated that genetic factors are involved. However, the majority of cases of ONFH are sporadic and various studies have demonstrated that differences in the study design and/or the ethnic groups analyzed leads to different results. The present study performed one of the first genome-wide association studies to identify genetic loci that may increase the risk of idiopathic ONFH. In total, 217 patients with idiopathic ONFH and 217 control samples, without ONFH, were genotyped using Axiom™ chips. Following quality control, 509,886 single-nucleotide polymorphisms (SNPs) were included in the association analysis to identify genetic variants that may influence susceptibility to idiopathic ONFH. The lowest P-value identified by the current study was for an association with rs220324 (P=3.57×10-7), an SNP that is located near to the uromodulin-like 1 gene region on chromosome 21q22.3, although none of the SNPs reached the traditional genome-wide significance level of 5×10–8. However, the DnaJ heat shock protein family (Hsp40) member C6 (DNAJC6) locus, a region between 65.37 and 65.67 Mb located on chromosome 1p31.3, harbored a cluster of SNPs that were associated with idiopathic ONFH at a significance level of P<1×10–5. Four variants, rs10493374, rs12032616, rs17127529 and rs6679032, with marginal associations were located in and around the DNAJC6 locus and were in strong linkage disequilibrium with each other. In conclusion, the current study did not identify any SNPs that were associated with idiopathic ONFH at a genome-wide significance level, however, the results suggest that future studies should investigate the effects of SNPs in the DNAJC6 gene on the idiopathic ONFH risk.

Objective: To determine the distribution of referred hip pain in patients with idiopathic osteonecrosis of the femoral head (ION).Methods: We prospectively documented 119 hips in 90 patients with ION (mean age 51 years). Patients identified the location of pain originating in their hip on a drawing of the body. Osteoarthritis of the hip (OA) was used as a historical cohort.Results: Referral of pain originating from the hip in patients with ION was 93% (111 hips) to the groin, 68% (81 hips) to the knee, 36% (43 hips) to the anterior thigh, 34% (40 hips) to the buttock, 18% (22 hips) to the lower leg, 9% (11 hips) to the greater trochanter, and 8% (9 hips) to the low back. About 97% (115 hips) of pain was located in the hip region (groin, buttock, and greater trochanter) and 77% (92 hips) showed referred pain (anterior thigh, knee, lower leg, and low back). Pain from ION was significantly more frequent in the knee and lower leg, but significantly less frequent in the lower back than pain from OA.Conclusion: We should be aware of ION masquerading as pain in the knee or anterior thigh.

Idiopathic osteonecrosis of the femoral head (idiopathic ONFH) is an ischemic disorder resulting in necrosis of the subchondral bone of the femoral head. COL2A1 mutations, including c.3508G>A, have been reported to be involved in its etiology. However, the etiological role of COL2A1 mutations in idiopathic ONFH remains controversial, because the pathology of idiopathic ONFH is ischemic necrosis, not epiphyseal dysplasia usually seen in the diseases caused by COL2A1 mutations. The purpose of this study is to examine whether COL2A1 mutations have causal relation with idiopathic ONFH or not. We recruited 1,451 Japanese patients with idiopathic ONFH, including steroid-, alcohol-, and neither steroid nor alcohol-associated (neither-associated) ONFH. The diagnosis was based on the criteria of the Japanese Research Committee on idiopathic ONFH of the Ministry of Health, Labour and Welfare. By whole-exome sequencing, entire COL2A1 coding regions and flanking introns were analyzed in 49 neither-associated ONFH patients. In addition, the c.3508G>A mutation of COL2A1 was checked in all idiopathic ONFH patients using the invader assay. Whole-exome sequencing did not detect any COL2A1 mutations in the 49 patients. The c.3508G>A mutation was not found in any of the 1,451 patients. In conclusion, COL2A1 is unlikely to cause idiopathic ONFH. Epiphyseal dysplasia of the femoral head caused by COL2A1 mutations may radiographically mimic idiopathic ONFH. COL2A1 mutations should prompt clinical re-evaluation of the patient's phenotype. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:768-774, 2017.

Osteonecrosis of the femoral head has a host of etiologies. However, in 30% of the cases, no underlying etiology is identified and the process is considered "idiopathic." Our hypothesis was that osseous anatomical abnormalities might be frequently found in patients with femoral head osteonecrosis. A retrospective, 1:2 matched, case-control study was undertaken to compare ninety patients with idiopathic osteonecrosis who had undergone lower-limb computed tomography (CT) prior to undergoing total hip arthroplasty with 180 control patients matched for age, sex, and body mass index (BMI) who had undergone lower-limb computed tomography scanning at our radiology department for any reason except articular or bone disease. Preoperative CT scans were performed for all patients to evaluate femoral offset, femoral neck-shaft angle, femoral neck version, femoral head diameter, acetabular coverage estimated with use of the lateral center-edge angle, acetabular version, and acetabular diameter. The mean age was forty-five years, 69% of the patients were men, and the mean BMI was 26 kg/m2. Anatomical "abnormalities" associated with osteonecrosis were determined to be a femoral neck-shaft angle of <129° (likelihood ratio [LR] = 3.6), femoral neck version of >17° (LR = 3.8), a lateral center-edge angle of <32° (LR = 5.7), and acetabular version of <19° (LR = 1.38). A combination of three of the four anatomical "abnormalities" was found in 73% of the patients with osteonecrosis but only 11% of the control cases (LR = 6). This 1:2 matched, anatomical study suggests that acetabular and femoral anatomical factors, in isolation or combination, can be found in a large percentage of cases of "idiopathic" osteonecrosis. In this study, isolated or combined abnormalities of the hip were observed in the group of patients with osteonecrosis. Those elements may help surgeons to understand the process underlying idiopathic osteonecrosis and thus require further investigation.

A Case of Bilateral Idiopathic Osteonecrosis of the Femoral Head Treated by Bilateral Minimally Invasive Total Hip Arthroplasty (MIS-THA) in a Patient with Liver Cirrhosis Performed in a One-day Session

Background: Management of idiopathic osteonecrosis of the femoral head with arthritis in the young age group is controversial. Total hip replacement is considered the gold standard for management of arthritic hip. However, the longevity of the prosthesis and the number of future revisions are still the main source of concern about total hip replacement in this age group. The use of ceramic on ceramic bearing couples improved the longevity of the prosthesis. Patients and methods: In the period from June 2010 – to December 2012, 18 patients (22 hips) with idiopathic osteonecrosis of the femoral head were treated by cement less ceramic on ceramic total hip replacement. This study was done at Zagazig university hospitals. The mean age was 29.5 years old. There were 14 males and 4 females. The mean follow up period was 48.4 months. All cases had idiopathic osteonecrosis of the femoral head stage III and IV according to Ficat classification. The functional results were evaluated according to Harris hip score. The radiological results of the femoral and acetabular components following surgery were evaluated according to the criteria of Lins et al and Engh et al., respectively. Results: The mean Harris hip score improved from 39.36 (range 30-56) preoperatively to 89.59 (range 68-96) in the final controls (p

Idiopathic osteonecrosis of femoral heads resulting in bilateral total hip replacement: report of a suspected malingerer

Treatment with low-intensity pulsed ultrasound on idiopathic osteonecrosis of the knee

We studied patients who received THR using VerSys Hip System and who could be followed-up more than 3 years. Clinical records were used to retrospectively identify patient characteristics, which included age, gender, disease requiring THR, preoperative and postoperative pain score of the Japan Orthopaedic Association scoring system, range of motion in flexion and abduction, operating time, intraoperative complication, and additional operation or revision surgery. Additionally, we investigated the loosening and alignment of implants from X-ray films. Ninety-one patients and 108 hip joints were investigated. Subjects were 11 males and 97 females (mean age, 64.6 years). Mean follow-up period was 6.9 years. Reasons for requiring THR were as follows: secondary osteoarthritis, 87 joints; idiopathic osteonecrosis of the femoral head, 16 joints; rapidly destructive coxarthrosis, 4 joints; and idiopathic ossification of the labrum, 1 joint. Mean operating time was 166 minutes. A total of 11 intraoperative fractures occurred, and wiring was performed in 3 of those cases. Adverse events pertaining to the surgery were limited; however, another adverse event was that 1 case resulted in intraoperative perforation of femoral cortex, for which a revision surgery was performed. There was no dislocation. Pain score using the Japan Orthopaedic Association scoring system and range of motion tests showed statistically significant improvement following THR. At the final follow-up, although no loosening of femoral implants was observed, the loosening of acetabular component was seen in 1 case. Varus insertion of femoral implant was recognized in 40 joints. Moreover, the average inclination angle of acetabular implants was 52.2 degrees. These data suggest that patients receiving VerSys Hip System uncemented THR demonstrate favorable results pertaining pain score and range of motion. However, high rate of intraoperative fracture and malalignment of implants, which may be at a risk of dislocation and/or polyethylene wear in future, suggests that this implant technique requires improvement.

Although this effect is not widely recognized, testosterone therapy can interact with thrombophilia, causing osteonecrosis. In 12 men and 4 women who had idiopathic osteonecrosis a median of 6 months after the onset of testosterone therapy, the authors examined the interaction between testosterone therapy and previously undiagnosed thrombophilia. The authors hypothesized that patients who had osteonecrosis after starting testosterone therapy were more likely than 110 normal control subjects or 48 patients who had osteonecrosis and were not receiving testosterone therapy to have thrombophilia. Measures of thrombophilia included Factor V Leiden, prothrombin, PAI-1 gene mutations, Factor VIII, Factor XI, anticardiolipin antibody immunoglobulin G or immunoglobulin M, and homocysteine values. In 10 cases, osteonecrosis occurred 6 months or less after the onset of testosterone therapy, and in all 16 cases, it occurred after a median of 6 months of testosterone therapy. Of the 16 cases, 5 (31%) were Factor V Leiden heterozygotes vs 2 of 109 (2%) healthy control subjects (P=.0003) and 4 of 48 patients who had osteonecrosis and were not receiving testosterone therapy (P=.04). Of the 16 cases, 4 (25%) had high (>150%) Factor VIII levels vs 7 of 103 (7%) healthy control subjects (P=.04), and 3 (19%) had high (>150%) Factor XI levels vs 3 of 101 (3%) healthy control subjects (P=.03). Of the 16 patients with osteonecrosis, 14 (88%) had at least 1 abnormal procoagulant value (of the 8 measured) vs 47 of 110 (43%) healthy control subjects (P=.0009). Of the 5 men whose serum estradiol level was measured while they were receiving testosterone therapy, this level was high (≥42.6 pg/mL) in 4. When testosterone therapy is given to patients with thrombophilia, they are at increased risk for osteonecrosis.

IntroductionReports of dislocation after bipolar hemiarthroplasty (BHA) abound in literature, and several studies have mentioned the factors that are associated with an increased risk of dislocation. However, there is no report detailing the pattern of impingement in BHA and how femoral antetorsion can affect the range of motion (ROM) after BHA.PurposeThe purpose of this study was to evaluate the pattern of impingement in BHA and whether femoral antetorsion affects the ROM after BHA using three-dimensional (3D) dynamic motion analysis.MethodsUsing the computed tomography (CT) data of 60 patients (60 hips), including 31 men and 29 women who underwent BHA for the treatment of idiopathic osteonecrosis (ION) of the femoral head, we calculated the antetorsion of the femoral neck, ROM of flexion (Flex), internal rotation (Int-R), and external rotation (Ext-R) using a CT-based 3D simulation software. We evaluated the pattern of impingement and the relationship between femoral antetorsion and ROM in BHA. As for the implant position in the 3D simulation software, the anteversion of the femoral implant was set to be the same as the natural antetorsion of the femoral neck and neck length was set to be the standard neck in all cases.ResultsThis study revealed the mechanism of impingement in BHA: (1) bone to bone impingement and (2) implant to bone impingement. We found a significant decrease in the ROM of Flex and Int-R inversely proportional to the femoral antetorsion. In patients with lower femoral antetorsion, the ROM of Flex and Int-R decreased due to bony impingement (the anterior great trochanteric region of the femur impinges on the anteroinferior edge of the anteroinferior iliac spine). Whereas, high anteversion of the femoral implant may decrease the ROM of Ext-R; however, our results also showed that even the lowest ROM of Ext-R with 10° hip extension was over 40°.ConclusionsWe demonstrated that lower femoral antetorsion substantially affects the ROM of Flex and Int-R due to bony impingement. For these patients, there should be consideration given to retaining femoral “anterior offset” in BHA.

Evaluation on Early Stages of Idiopathic Osteonecrosis of the Knee by Non-Surgical Treatment

To study a modified porous tantalum technique for the treatment of osteonecrosis of the femoral head. The porous tantalum rod was combined with endoscopy, curettage, autologous bone grafting and use of bone marrow aspirates from iliac crest aspiration in 49 patients (58 hips) with a mean age of 38 years. The majority of the patients had idiopathic osteonecrosis, followed by corticosteroid-induced osteonecrosis. Thirty-eight hips were of Steinberg stage II disease and 20 hips were of stage III disease. Patients were followed for 5 years and were evaluated clinically with the Merle D'Aubigne and Postel score and radiologically. The primary outcome of the study was survival based on the conversion to total hip arthroplasty (THA). Secondary outcomes included deterioration of the osteonecrosis to a higher disease stage at 5 years compared to the preoperative period and identification of factors that were associated with survival. The Kaplan-Meier survival analysis was performed to evaluate the survivorship of the prosthesis, and the Fisher exact test was performed to test associations between various parameters with survival. No patient developed any serious intraoperative or postoperative complication including implant loosening or migration and donor site morbidity. During the 5-year follow up, 1 patient died, 7 patients had disease progression and 4 hips were converted to THA. The 5-year survival based on conversion to THA was 93.1% and the respective rate based on disease progression was 87.9%. Stage II disease was associated with statistically significant better survival rates compared to stage III disease (P = 0.04). The comparison between idiopathic and non-idiopathic osteonecrosis and between steroid-induced and non-steroid-induced osteonecrosis did not showed any statistically significant difference in survival rates. The clinical evaluation revealed statistically significantly improved Merle d'Aubigne scores at 12 mo postoperatively compared to the preoperative period (P < 0.001). The mean preoperative Merle d'Aubigne score was 13.0 (SD: 1.8). The respective score at 12 mo improved to 17.0 (SD: 2.0). The 12-mo mean score was retained at 5 years. The modified porous tantalum rod technique presented here showed encouraging outcomes. The survival rates based on conversion to THA are the lowest reported in the published literature.

In 6 patients with familial thrombophilia (5 Factor V (FV) Leiden heterozygotes, 1 with resistance to activated protein C (RAPC)), we prospectively assessed whether continuous longterm (4–16 years) anticoagulation would prevent progression of idiopathic osteonecrosis (ON), ameliorate pain, and facilitate functional recovery. Four men and 2 women (9 hips, 8 Ficat stage II, 1 stage I) were anticoagulated with enoxaparin (60 mg/day) for 3 months and subsequently with Coumadin, Xarelto, or Pradaxa, warranted by ≥2 prior thrombotic events. Anticoagulation was continued for 4, 4, 9, 13, 13, and 16 years, with serial clinical and X-ray follow-up. On 4–16-years anticoagulation, 9 hips in the 6 patients (8 originally Ficat II, 1 Ficat I) remained unchanged, contrasted to untreated ON Ficat stage II, where 50%–80% of hips progress to collapse (Ficat stages III-IV) within 2 years after diagnosis. Within 3, 3, 3, 9, and 16 months after starting anticoagulation, 5 patients became pain-free and remained asymptomatic throughout follow-up; the 6th patient required Percocet for pain. There were no significant bleeding episodes. Long term (4–16 years) anticoagulation initiated in Ficat stages I-II of idiopathic hip ON in patients with FV-RAPC changes the natural history of ON, stopping progression, resolving pain, and restoring function.

Spontaneous osteonecrosis of the knee presents with acute onset of severe pain in elderly patients, usually females, without a history of trauma. Originally described as idiopathic osteonecrosis, the exact etiology is still debated. Evidence suggests that an acute fracture occurs as a result of chronic stress or minor trauma to a weakened subchondral bone plate. We report one case of spontaneous osteonecrosis of medial femoral condyle in a 71-year-old female with no evidence of trauma involving a 4 cm weight bearing surface. According to Koshino this case can be classified as stage II. Various types of treatment have been reported, including conservative treatment with physical exercise and nonsteroidal anti-inflammatory drugs, arthroscopy alone, arthroscopy and drilling, valgus high tibial osteotomy, and total knee arthroplasty. Total knee arthroplasty was considered the appropriate management strategy in this case.

Idiopathic osteonecrosis of the femoral head in the contralateral hip newly developed two years after unilateral hip replacement. A case report

Rehabilitation Program After Mesenchymal Stromal Cell Transplantation Augmented by Vascularized Bone Grafts for Idiopathic Osteonecrosis of the Femoral Head: A Preliminary Study